ABSTRACT
Background Fibroblast growth factor 23 (FGF23) is a recently discovered bone-derived regulator of vitamin D metabolism and phosphate homeostasis. It inhibits phosphate reabsorption and calcitriol production by the kidney. Myelomeningocele (MMC) remains the most severe form of neural tube defects involving serious locomotor disability, osteoporosis and pathologic fractures. We aimed to investigate the influence of vitamin D replacement therapy on serum FGF23 concentration in children with MMC and compare the results with healthy participants. Methods This prospective analysis was conducted on 16 children with MMC and 20 healthy children. Serum FGF23 levels were measured; for the studied group, before and after vitamin D replacement therapy with cholecalciferol (vitamin D3). The children's medical charts were analyzed to determine age, sex, anthropometric measurements, calcium and phosphate, cholecalciferol and renal function parameters. Results There were significant differences in vitamin D and FGF23 serum concentrations between the studied groups. The median vitamin D levels in the MMC group increased during replacement therapy (7 vs. 18.5 ng/mL, p = 0.29) in comparison to the median of 25.5 ng/mL in the control group. In MMC children we found a significant decrease in median serum FGF23 after vitamin D replacement therapy (from 42.1 to 0 RU/mL, p < 0.001). FGF23 correlated positively with albumin, serum and urine phosphate levels and negatively with alkaline phosphatase. Conclusions 1. Serum concentration of FGF-23 is increased in MMC children in comparison to a healthy control group. 2. Vitamin D replacement therapy decreases FGF23 concentrations in MMC children, although further studies are still warranted to gain detailed insight on the FGF23 in the MMC population. 3. Children with MMC present vitamin D deficiency. Nutrition supplemented with low doses of cholecalciferol (vitamin D3) (intakes reaching recommended daily allowances) was insufficient to correct 25(OH)-D level in that population of patients.
Subject(s)
Biomarkers/blood , Dietary Supplements , Fibroblast Growth Factors/blood , Meningomyelocele/blood , Vitamin D/administration & dosage , Vitamins/administration & dosage , Adolescent , Case-Control Studies , Child , Child, Preschool , Female , Fibroblast Growth Factor-23 , Follow-Up Studies , Humans , Infant , Male , Meningomyelocele/drug therapy , Meningomyelocele/pathology , Prognosis , Prospective StudiesABSTRACT
OBJECTIVE: To determine a solution capable of discerning adipose versus nervous tissue, to aid in surgical separation of the adipose tissue which appears to be visually indistinguishable from nervous tissue in lipomyelomeningoceles (LMMs). METHODS: The following solutes (in normal saline) were investigated, both at 25 and 37 degrees C: beta-carotene, vitamin D, vitamin E, lecithin, hydrogen peroxide, lipase, protease, hyaluronidase, partially purified collagenase, purified collagenase, trypsin, trypsin plus purified collagenase and non-solute-containing saline (control). Each solution was applied to a pediatric lipoma to determine gross effects over a period of approximately 30 min. If a solution appeared to affect the adipose tissue grossly, studies of functional in vivo sensory evoked and spontaneous potentials using that particular solution were conducted upon sheep spinal cord, nerve roots, dura and peripheral nerve. Additionally, histological studies were conducted to determine the effect of that solution upon adipose tissue, spinal cord, myelin, dura and nerve roots. RESULTS: Of all solutions investigated, partially purified collagenase type 1 (T1C; Lot M0M4322, Code CLS-1, Worthington Biochemical Corporation, Lakewood, N.J., USA) at 37 degrees C was the most successful in grossly altering the consistency and appearance of adipose tissue. This change was more apparent over 20-30 min following application of the solution to the adipose tissue. Solutions not containing T1C did not show appreciable results; purified collagenase plus trypsin did not appear comparable or superior to T1C. No significant histological or functional change was noted when comparing the spinal cord, nerve rootlets, myelin, dura or peripheral nerve from the T1C-treated group versus normal (untreated) control groups. CONCLUSION: T1C appears to be a potentially effective solution for application during LMM surgery in the acute setting, and such use of an adjunct solution may significantly aid in the safe surgical resection of LMMs. Pending further research, this technique may be applied for other indications which require discernment or alteration of adipose versus nervous tissue.
Subject(s)
Adipose Tissue/drug effects , Adipose Tissue/pathology , Lipoma/pathology , Lipoma/surgery , Meningomyelocele/pathology , Meningomyelocele/surgery , Nerve Tissue/drug effects , Nerve Tissue/pathology , Pharmaceutical Solutions/chemistry , Pharmaceutical Solutions/pharmacology , Adipose Tissue/surgery , Chemotherapy, Adjuvant , Child , Collagenases/chemistry , Collagenases/pharmacology , Collagenases/therapeutic use , Humans , In Vitro Techniques , Lipoma/drug therapy , Meningomyelocele/drug therapy , Nerve Tissue/surgery , Pharmaceutical Solutions/therapeutic use , Time FactorsABSTRACT
28 myelomeningocele patients (aged 2-30 years) with clinical symptoms of acute UTI participated in this open uncontrolled clinical trial at the Orthopedic University Hospital of Heidelberg (Dir.: Prof. Dr. H. Cotta). 4 patients were treated with 200 mg cefixime tablets bid, 24 patients received 4 mg/kg body weight cefixime suspension bid, according to age and weight of the patients. The duration of treatment was 6-10 days. Clinical and microbiological examinations were carried out before therapy as well as 1 day and 5 to 9 days after the end of treatment. The data of 25 patients could be evaluated for bacteriological and clinical efficacy. 5-9 days after treatment in 22 patients (88%) complete recovery was stated. In 3 patients a reinfection occurred. In 24 patients (96%) the baseline pathogens were eliminated under cefixime therapy. 5-9 days after the end of treatment in 3 patients reinfection was observed. Clinical side effects could be detected in 1 patient (vomiting). These results indicate that the oral cephalosporin cefixime is efficient and well tolerated in complicated UTI of myelomeningocele patients.